Author + information
- Wu Shaohong,
- Meiqing Cheng,
- Zuanan Chen and
- Zuofeng Xu
Infantile high cardiac output due to multiple hepatic hemangiomas is a common cause of congestive heart failure but rare attention to original diagnosis. To report the cardiac insufficiency of infancy due to multiple hepatic hemangiomas and review the management strategies of this disease.
A 54-day-old female infant was admitted to our hospital because of cough, shortness of breath and feeding difficulties lasting for 2weeks.A general physical examination revealed several red rash(0.3-1.5cm)on limbs, libs and perioral cyanosis while crying. Her heart rate was between 150 and 160bpm. The liver was palpated approximately 5cm and the spleen was 3cm below the costal margin. Echocardiography revealed severe pulmonary hypertension(PASP=100mmHg), right heart enlargement, and normal left ventricular ejection fraction (EF74%). Abdominal ultrasonography(USG)found hepatomegaly and innumerable hepatic hemangiomas(0.8-2.2cm). Laboratory data showed anemia(Hb85g/l). Anti heart failure treatment were administered with digoxin,frusemide, spironolactone and hydrochlorothiazide. Other treatment including oxygen and red blood cell transfusion were supplied. While the symptoms was not improved by those treatment.USG showed the lesions in liver was enlarged(0.5-3.2cm). Considering the infantile hepatic hemangioma(IHH)was probably associated with heart failure,propranolol(2.5mg tid)and prednisone(2mg/kg/d)was added.
After the above treatment measures, the clinical manifestation of this patient was improved gradually after 3weeks. The patient was followed up after 5months, rechecked USG revealed decreased size of hepatic hemangioma(0.5-1.6cm)and pulmonary artery pressure decreased(PASP=42mmHg).
With the special pathological and physiological characteristics that often merged with arteriovenous fistula,IHH may influence heart function and lead to cardiac dysfunction, mainly manifested as right heart failure, and cause 90% mortality rate. And cardiac insufficiency can aggravate hemangioma inversely because of body circulation reflux disorder. The diagnosis of IHH mainly relies on USG, computed tomography or magnetic resonance imaging. The evaluation of patients suspected this disease remains challenging because symptoms or signs are not unique. We aim to emphasize the vital importance of experience that USG should be used more often for screening patients of congenital hepatic hemangiomas leading to congestive heart failure, when the etiology of severe cardiac insufficiency failure cannot be explain in infancy and cardiac function deteriorate despite optimal medical therapy. In conclusion, appropriate treatment requires early diagnosis to reduce mortality rate. Now beta-blockers(initial dose 1mg/kg/d, and taget dose2.5-3.0mg/kg/d)are recommended for the optimal choice because it is highly effective and well tolerated with few serious side effects. It should prompt consideration of combination therapy with glucocorticoid (prednisone2-3mg/kg/d divied three times daily for 2-3weeks, with a 10day taper)in cases with serious complications.